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Amino Acids

, Volume 12, Issue 2, pp 157–166 | Cite as

Plasma carnitine status - a prognostic factor in children with dilated cardiomyopathy

  • M. Marx
  • M. Skyllouriotis
  • E. Legenstein
  • E. Proll
  • M. Wimmer
Full Papers

Summary

Objective - Dilated cardiomyopathy is a rare disorder in childhood that results in a high mortality. The aim of our study was to evaluate the prognostic relevance of the individual plasma carnitine status in children with dilated cardiomyopathy.

Methods - In 26 patients plasma carnitine concentrations were determined before and after 6 and 12 months of L-carnitine treatment. According to the plasma short chain acyl-carnitine/free carnitine ratio (AC/FC) at the first presentation children were divided into two groups.

Results - In group 1 (AC/FC < 0.4) the median time from diagnosis until death was 35.8 months, the cumulative survival rate was 84% after 2 years. In group 2 (AC/FC > 0.4) median time from diagnosis until death was 8 months, the cumulative survival rate was 50% at 2 years (p < 0.05).

Dividing both groups into survivors and nonsurvivors in group 2 a significantly higher AC/FC ratio in the nonsurvivors could be found (survivors 0.78 v 1.3 in nonsurvivors). A significant improvement of left ventricular function 6 and 12 months after presentation and after starting L-carnitine treatment could only be documented in the surviving patients of group 2.

Conclusion - The individual plasma carnitine status in children with dilated cardiomyopathy may serve as a risk factor for survival.

Keywords

Amino acids Dilated cardiomyopathy Infants and children Plasma carnitine 

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References

  1. Akagi T, Benson LN, Lightfoot NE, Chin K, Wilson G, Freedom RM (1991) Natural history of dilated cardiomyopathy in children. Am Heart J 121: 1502–1506PubMedGoogle Scholar
  2. Battistella A, Vergani L, Donzelli F, Rubaltelli FF, Angelini C (1980) Plasma and urine carnitine levels during development. Pediat Res 14: 1379–1381PubMedGoogle Scholar
  3. Berko BA, Swift M (1987) X-linked dilated cardiomyopathy. N Engl J Med 316: 1186–1189PubMedGoogle Scholar
  4. Bohles H, Evangeliou A, Bervoets K (1994) Carnitine esters in metabolic disease. Eur J Pediatr 153 [Suppl] 1: 57–61Google Scholar
  5. Brass EP, Hoppel CL (1980) Relationship between acid-soluble carnitine and coenzyme A pools in vivo. Biochem J 190: 459Google Scholar
  6. Cederblad G, Linstedt S (1972) A method for the determination of carnitine in the picomole range. Clin Chem Acta 37: 235–240Google Scholar
  7. Chen SC, Nouri S, Balfour I, Jureidini S, Appleton S (1990) Clinical profile of congestive cardiomyopathy in children. J Am Coll Cardiol 15: 189–193PubMedGoogle Scholar
  8. Ciszewski A, Bilinska ZT, Lubiszewska B, Ksiezycka E, Poplawska W, Michalak E, Walczak E, Walczak F, Ruzyllo W (1994) Dilated cardiomyopathy in children: clinical course and prognosis. Pediatr Cardiol 15: 121–126PubMedGoogle Scholar
  9. Drexler H (1992) Skeletal muscle failure in heart failure. Circulation 85: 1621–1623PubMedGoogle Scholar
  10. Engel AG (1980) Possible causes and effects of carnitine deficiency in man. In: Frankel RA, McGarry D (eds) Carnitine biosynthesis, metabolism and function. Academic Press, New York, pp 271–285Google Scholar
  11. Engel AG, Angelini C (1973) Carnitine deficiency of human skeletal muscle with associated lipid storage myopathy: a new syndrome. Science 179: 899–902PubMedGoogle Scholar
  12. Feigenbaum H (1986) Echocardiography. Perimed, Erlangen, pp 148–151Google Scholar
  13. Friedman RA, Moak JP, Garson A Jr (1991) Clinical course of idiopathic dilated cardiomyopathy in children. J Am Coll Cardiol 18: 152–156PubMedGoogle Scholar
  14. Fuster V, Gersh BJ, Giccliani ER, Tajik AJ, Brandenberg RO, Frye RL (1981) The natural history of idiopathic dilated cardiomyopathy. Am J Cardiol 47: 525–531PubMedGoogle Scholar
  15. Greenwood M (1926) The errors of sampling of the survivorship tables: Appendix 1. In: Reports on Puplic Health and Statistical Subects. no 33. London: His Majesty's Stationary OfficeGoogle Scholar
  16. Griffin ML, Hernandez A, Martin ThC, Goldring D, Bolman RM, Spray ThL, Strauss AW (1988) Dilated cardiomyopathy in infants and children. J Am Coll Cardiol 11: 139–144PubMedGoogle Scholar
  17. Hütter JF, Piper HM, Spieckermann PG (1985) Effect of fatty acid oxidation on efficiency of energy production in rat hearts. Am J Physiol 249: 723–728Google Scholar
  18. Kaplan EL, Meier P (1958) Nonparametric estimation from incomplete observations. J Am Stat Assoc 53: 457–481Google Scholar
  19. Lewis AB, Chabot M (1991) Outcome of infants and children with dilated cardiomyopathy. Am J Cardiol 68: 365–369PubMedGoogle Scholar
  20. Lohninger A, Kaiser E, Legenstein E, Staniek H (1987) Carnitine, metabolism and function. In: Kaiser E, Lohninger A (eds) Carnitine - Its role in lung and heart disorders. Karger, Basel, pp 1–25Google Scholar
  21. Magnusson G, Isberg B, Karlberg KE, Sylven C (1994) Skeletal muscle strength and endurance in chronic congestive heart failure secondary to idiopathic dilated cardiomyopathy. Am J Cardiol 73: 307–309PubMedGoogle Scholar
  22. Mancini DM, Wilson JR, Bolinger L, Li H, Kendrick K, Chance B, Leigh JS (1994) In vivo magnetic resonance spectroscopy measurement of deoxymyoglobin during exercise in patients with heart failure: demonstration of abnormal muscle metabolism despite adequate oxygenation. Circulation 90: 500–508PubMedGoogle Scholar
  23. Opie LH (1979) Role of carnitine in fatty acid metabolism of normal and ischemic myocardium. Am Heart J 97: 375–378PubMedGoogle Scholar
  24. SAS User's Guide (1985) Statistics. Version 5 Edition: SAS Institute Inc. Cary, NCGoogle Scholar
  25. Shug AL (1975) Acyl-CoA inhibition of adenine nucleotide translocase in ischemic myocardium. Am J Physiol 228: 689–692PubMedGoogle Scholar
  26. Tripp ME, Shug AL (1984) Plasma carnitine concentrations in cardiomyopathy patients. Biochem Med 32: 199–206PubMedGoogle Scholar
  27. Winter SC, Szabo-Aczel S, Curry CJR, et al. (1987) Plasma carnitine deficiency: clinical observations in 51 patients. AJDC 141: 660–665PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1997

Authors and Affiliations

  • M. Marx
    • 1
  • M. Skyllouriotis
    • 1
  • E. Legenstein
    • 2
  • E. Proll
    • 1
  • M. Wimmer
    • 1
  1. 1.Abteilung Pädiatrische KardiologieUniversitätsklinik für Kinder- und JugendheilkundeAKH WienAustria
  2. 2.Institut für Medizinische Chemie, Universität WienViennaAustria

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